Objective: To report the diagnosis, radiologic findings, and therapy of a 51-year-old female with systemic lupus erythematosus (SLE) who, while on hydroxychloroquine maintenance therapy, presented with a junctional scotoma indicative of chiasmal disease. This visual loss developed after she had been tapered off corticosteroids.
Materials and methods: An interventional case report of a female that was given acute therapy with 1-gram daily of intravenous methylprednisolone sodium succinate for 5 days, followed by maintenance methotrexate and a slow taper of oral prednisone. Magnetic resonance imaging (MRI) scans, visual acuity, color vision, and threshold visual fields were performed.
Results: The MRI scan showed chiasmal involvement, which may occur in SLE in absence of any other evidence of systemic activity. Therapy led to visual function returning to 20/20 OD and 20/20 OS, with normal Ishihara plates OU and only minimal paracentral depressions OU. She has been able to be weaned off prednisone while on methotrexate maintenance.
Conclusions: Chiasmal involvement may occur in SLE in absence of any other evidence of systemic activity. Maintenance with hydroxychloroquine may not be adequate to prevent this rare cause of visual loss in SLE. Aggressive therapy of chiasmal involvement in SLE, even when the visual loss is profound, may lead to visual restoration, which was virtually complete in this case. Methotrexate may be an alternate agent for patients who break through with optic neuropathy while on hydroxychloroquine.